Brugada syndrome (BrS), an inherited arrhythmogenic disease first described in 1992, is characterized by ST segment elevations on the electrocardiogram in the right precordium and by a high occurrence of arrhythmias including the life-threatening ventricular tachycardia/fibrillation. Knowledge of the underlying mechanisms of formation of arrhythmogenic substrate in BrS is essential, namely for the risk stratification of BrS patients and their therapy which is still restrained almost exclusively to the implantation of cardioverter/defibrillator. In spite of many crucial findings in this field published within recent years, the final consistent view has not been established so far. Hence, BrS described 20 years ago remains an actual topic of both clinical and experimental studies. This review presents an overview of the current knowledge related to the pathogenesis of BrS arrhythmogenic substrate, namely of the genetic basis of BrS, functional consequences of mutations related to BrS, and arrhythmogenic mechanisms in BrS.

Department of Physiology Faculty of Medicine Masaryk University Kamenice 5 625 00 Brno Bohunice Czech Republic

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Lessons on the non-linear path of medical progress and biological complexity from mouse models of the Brugada syndrome